Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency.
Conclusion: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients.
What is known:
• Auditory neuropathy dyssynchrony spectrum disorder (ANSD) is a particular form of hearing loss, mostly encountered in neonatal intensive care unit (NICU) graduates.
• Little data are available on the prevalence and risk factors for ANSD in healthy newborns.
What is new:
• The estimated prevalence of ANSD in healthy newborns is 0.09/1000 live births.
• In about half of the healthy newborns with ANSD, a structural abnormality was detected on magnetic resonance imaging of the posterior fossa/brain.
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Auditory brain stem responses
Auditory steady state responses
Cochlear nerve deficiency
Magnetic resonance imaging
Normal hearing level
Neonatal intensive care unit
Transient evoked otoacoustic emissions
Universal neonatal hearing screening
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The authors would like to thank Luc Stappaerts from Kind and Gezin who provided the data on the UNHS screening for Flanders.
All authors contributed to data collection, interpretation of the data and preparation of the manuscript.
None of the authors received any financial support or funding for the present study.
Conflict of interest
An Boudewyns declares that she has no conflict of interest.
Frank Declau declares that he has no conflict of interest.
Jenneke van den Ende declares that she has no conflict of interest.
Anouk Hofkens declares that she has no conflict of interest.
Sara Dirckx declares that she has no conflict of interest.
Paul van de Heyning declares that he has no conflict of interest.
The study was performed in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed consent was obtained from the caregivers of all individual infants included in the study.
Communicated by Peter de Winter
Appendix: Cochlear microphonic
Appendix: Cochlear microphonic
The cochlear microphonic is a preneural response from the cochlear outer hair cells. The presence of a cochlear microphonic at or below a sound level that does not evoke a recordable ABR is an indication for ANSD. The cochlear microphonic is a more robust criterion for the diagnosis of ANSD compared to transient evoked otoacoustic emissions because these may disappear with time or may be absent in cases where there is also a conductive component to the hearing loss (such as with middle ear effusion).
The recommended method to detect a CM is the use of separate, replicated runs of condensation and rarefaction polarity at a stimulus level of 80 dB nHL.
A CM is considered present when a waveform appears in the first few milliseconds of the tracing and is the only part of the waveform that reverses polarity from rarefaction to condensation. A control trial is then performed with the sound tube of the insert phone clamped to rule out transducer artefacts.
An example of a cochlear microphonic is provided below Fig. 3. More detailed information may be found at Lightfoot G (ed). 2011. Guidelines for cochlear microphonic testing. NHSP Clinical Group (http://www.thebsa.org.uk/wp-content/uploads/2015/02/CM_Guidance_v2_2109111.pdf).
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Boudewyns, A., Declau, F., van den Ende, J. et al. Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic. Eur J Pediatr 175, 993–1000 (2016). https://doi.org/10.1007/s00431-016-2735-5
- Auditory neuropathy spectrum disorder
- Neonatal hearing screening
- Hearing loss
- Auditory brain stem response